Congenital neurocristic cutaneous hamartoma with poliosis: A case report.

Neurocristic cutaneous hamartomas (NCH) are rare, pigmented skin lesions derived from the abnormal migration of neural crest cells. We report the case of a 57-year-old female with a congenital localized area of poliosis and underlying pigmented patch on her scalp. Analysis of 2 punch biopsies yielded features consistent with NCH. Histopathology revealed schwannian-differentiated spindle cells and melanocytic components in the dermis. The spindle cells stained positively for S-100 and the stroma showed a prominent CD34 staining. The melanocytes were positive for melanoma triple stain (HMB-45, Melan A, Tyrosinase) and S-100. As the presentation of NCH and focal poliosis has yet to be described, we will present a literature review of NCH and discuss common features and associations of poliosis.

Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case.

Fibroelastolytic papulosis is a rare, acquired fibroelastolytic disorder that presents clinically as white-to-yellow papules and plaques most commonly occurring on the neck of elderly patients. The term fibroelastolytic papulosis encompasses two closely related conditions previously described as pseudoxanthoma elasticum-like papillary dermal elastolysis (PDE) and white fibrous papulosis of the neck (WFPN). Here we present a case of a 78-year-old white female with a several-year history of numerous, asymptomatic 2-3 mm yellowish, non-follicular papules distributed symmetrically over the posterior neck, axillae, arm and antecubital fossae. Histopathologic examination revealed thickened and clumped elastotic fibers admixed with thick, sclerotic appearing collagen bundles in the mid and deep reticular dermis. Rare melanophages, loss of vertically oriented elastic fibers and scattered elastotic globes were noted in the papillary dermis. Based on the shared clinicopathologic features showed in this case, strong consideration should be made for the additional inclusion of papillary dermal elastosis as existing along the disease continuum of fibroelastolytic papulosis. This occurrence of fibroelastolytic papulosis shows unique histopathologic findings of pseudoxanthoma elasticum-like PDE, papillary dermal elastosis and WFPN, further supporting the theory that these entities exist as variants along the fibroelastolytic papulosis spectrum.